To evaluate the role of fluorine-18-fluorodeoxyglucose-positron emission tomography/computed tomography (18F-FDG-PET/CT) in staging and in detecting recurrence in uterine sarcomas (US), a systematic review was performed on PubMed and Medline, for studies reporting the diagnostic performance of18F-FDG-PET/CT, 16a-18F-fluoro-17b-estradiol (18F-FES)-PET/CT, 30-deoxy-30-[18F]-fluorothymidine (18F -FLT) -PET, conventional imaging (CT, magnetic resonance imaging and ultrasonography) in staging or in detecting recurrence/post therapy surveillance in US published up to October 2018. Out of 70 studies initially identified, 39 articles were chosen concerning the role of18F-FDG-PET/CT in staging and restaging in US, combined with other PET tracers or conventional imaging. To date, the preoperative evaluation of US is problematic. However, there is some evidence in favor of the use of18F-FDG-PET/CT in association with the study of certain clinicopathological factors, and metabolic parameters that showed good accuracy in the staging of US. This helps the stratification of patients according to the identified risk, contributing to avoid unnecessary surgery. Whereas, the majority of the studies included in this review article showed that 18F-FDG-PET/CT is an accurate method for the detection and localization of local and distant relapses in patients affected by US, especially when combined conventional imaging and immunohistochemical analysis. This combination had a good impact on clinical decision making of these patients. All the PET study results reported in this review have demonstrated the possible use of PET/CT in order to improve the assessment of this rare disease for the initial staging, therapeutic planning and subsequent follow-up. Given the small body of literature concerning this topic, further and larger studies are, therefore, an essential next step in confirming the value of this promising imaging tool.